Bickerstaff’s Brainstem Encephalitis (BBE) is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute. This condition has been named Bickerstaff’s brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the . Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by Edwin Bickerstaff in It may also affect the .

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Bickerstaff brainstem encephalitis – Wikipedia

In this condition, neuronal excitability can spread laterally to adjacent axons that may have an abnormally lowered threshold for firing as a result of demyelination.

The episodes increased in frequency so that they occurred up to hundreds of times a day. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: Bickerstaff’s brainstem encephalitis BBE is a rare post-infectious neurological disease characterized by the association of external ophthalmoplegia, ataxia, lower limb arreflexia, extensor plantar response and disturbance of consciousness drowsiness, stupor or coma.

About Contact Subscriptions Videos Privacy. Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss.

Focal Generalised Status epilepticus Myoclonic epilepsy. Wendy Terry Publisher wterry bmctoday. Common seropositivity between these two conditions highlights their pathophysiological and clinical similarities. Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections.


Randomised controlled trials are needed to establish the value of immunotherapies or other treatments.

There was no weakness. Oxford University Press is a department of the University of Oxford. Like some other autoimmune diseasesthe condition usually follows a minor infection, such as a respiratory tract infection or gastroenteritis.

Because coma continued, he was given 0. Needle electromyography showed positive sharp waves or fibrillation potentials in three patients on days 21— Needle electromyography was performed in six patients.

Treatment for Fisher syndrome, Bickerstaff’s brain stem encephalitis and related disorders

The precise incidence and prevalence of BBE in the United States and other Western nations is currently unknown, which can be attributed to the rarity of the disease and confusion and overlap with other Anti-Gq1b antibody syndromes.

An autopsy was performed. Diagnostic methods Diagnosis is based on the clinical findings, patient history, cerebrospinal fluid CSF analysis revealing raised protein levelsdetection of anti-GQ1b IgG antibodies not present in all patientsMRI studies revealing high-intensity abnormalities in the posterior fossa, white matter or thalami and neurophysiological examinations electroencephalogram and electromyography indicative of central nervous system and predominantly axonal involvement.

In addition to vrainstem episodes, the patient had minimal slurring of words when she first began to speak and a slight imbalance when she began to walk.

The brainstwm had transient and very frequent episodes of brainstem dysfunction during the recovery phase. Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis. A few days later, she noticed very frequent, stereotypic episodes lasting 1 to 10 seconds and occurring throughout the day; these episodes consisted of transient diplopia accompanied by dysarthria if talking and gait imbalance if walking.


Bickerstaff brainstem encephalitis

Deep sensation was frequently unimpaired despite profound ataxia. Blood study results showed no elevation of antibodies to bickerstafr viruses, Brucellaor Mycobacterium tuberculosis.

Roos, Soliven, and Baron.

The diagnosis of BBE is largely based on clinical features, though additional studies may aid in making an accurate diagnosis.

Bicksrstaff, each of these criteria fails to fit a substantial proportion of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis.

Orphanet: Bickerstaff brainstem encephalitis

Light reflexes were prompt. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more. In addition to Patient 2 described in the case reports, bickeerstaff patients died, one of sudden cardiopulmonary arrest and the other of haemorrhagic infarction, a complication in bicketstaff right cerebrum caused by candida meningitis. A remarkable feature of our patient was the presence of brief paroxysmal episodes of brainstem dysfunction that appeared after recovery of much of her neurological function.

In addition, we present pathological findings for a patient with BBE. His neurological signs disappeared in the order of ophthalmoplegia, ataxia, limb weakness and dysaesthesia. Data collection and analysis: